Reta Lila Weston Institute of Neurological Studies, UCL, 1 Wakefield Street, London, WC1N 1PJ, UK.
J Neurol. 2013 Apr;260(4):1009-13. doi: 10.1007/s00415-012-6745-6. Epub 2012 Nov 23.
Idiopathic normal pressure hydrocephalus (iNPH) is a poorly understood condition, which typically presents with the triad of balance impairment, urinary incontinence and subacute cognitive decline, while brain imaging shows a marked enlargement of the cerebral ventricles. Few patients with iNPH have come to post-mortem. We identified four patients from the Queen Square Brain Bank archival collection, who had received a diagnosis of iNPH during life, and reviewed their clinical, radiological and pathological characteristics. At post mortem examination, one patient had Parkinson's disease (PD) while the other three had progressive supranuclear palsy (PSP). All four had presented with pure akinesia with gait freezing, accompanied by unsteadiness and falls. An awareness that PSP or PD can mimic the clinical symptoms of iNPH may help to avoid invasive and futile cerebrospinal fluid shunting procedures.
特发性正常压力脑积水（iNPH）是一种发病机制尚不完全清楚的疾病，其典型表现为平衡障碍、尿失禁和亚急性认知功能下降三联征，而脑影像学显示脑室明显扩大。少数 iNPH 患者接受了尸检。我们从 Queen Square Brain Bank 存档资料中确定了 4 名生前被诊断为 iNPH 的患者，并回顾了他们的临床、影像学和病理学特征。尸检时，1 名患者患有帕金森病（PD），而其他 3 名患者患有进行性核上性麻痹（PSP）。这 4 名患者均表现为单纯的运动不能伴步态冻结，伴有站立不稳和跌倒。认识到 PSP 或 PD 可能模拟 iNPH 的临床症状，可能有助于避免侵入性和无效的脑脊液分流手术。
